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The following is a summary of “Management and outcomes of interstitial lung disease associated with anti-synthetase syndrome: a systematic literature review,” published in the July 2024 issue of Rheumatology by Kouranloo et al.
Anti-synthetase syndrome (ASS) is a chronic autoimmune disorder characterized by interstitial lung disease (ILD) as a prominent feature.
Researchers conducted a retrospective study summarizing treatments and outcomes of ILD in ASS.
They identified articles on ASS-ILD management and outcomes published through database searches (1946 to September 2023). Data extraction and screening were performed by 2 reviewers. Meta-analysis, using a random effects model, and paired t-tests were performed to assess changes in Pulmonary Function Tests (PFT) following treatment.
The result showed 10 articles, including 514 patients (67.8% female, mean age 52.4 years, SD 4.6). Baseline high-resolution computed tomography was reported for 447 patients (86.9%), with non-specific interstitial pneumonia being the most common pattern (49.2%, n=220). The most prevalent myositis-associated autoantibody was anti-Jo1 (48%), with 27.8% also having anti-Ro52 antibodies. Meta-analysis revealed pooled baseline estimates of 60.8% predicted forced vital capacity (FVC) (SE 2.1) and 49.8% diffusion capacity of lungs for carbon monoxide (DLco) (SE 3.5). After 1 year, pooled improvements in FVC and DLco were 14.1% (SE 3.1) and 15.1% (SE 2.8) from baseline, respectively. Paired t-tests showed significant overall improvements in FVC (P=0.007) and DLco (P=0.002). Patients treated with rituximab (RTX) exhibited a 12.2% improvement in FVC and a 2.9% increase in DLco after 1 year. Patients treated with cyclophosphamide (CYC) showed a 17% improvement in FVC and a 6.3% increase in DLco, with 28 deaths reported.
Investigators concluded that no definitive differences in treatment effectiveness for ASS-ILD were found, highlighting the need for more robust trials to reduce morbidity and mortality associated with the condition.
Source: academic.oup.com/rheumatology/advance-article-abstract/doi/10.1093/rheumatology/keae403/7724839